Infundibular Hemangioblastoma in a Patient with Neurofibromatosis Type 1: Case Report and Review of the Literature.

Abstract

Supratentorial hemangioblastomas are rare tumors, most commonly occurring in the sellar/suprasellar region, cerebrum, and ventricle. They are generally found in patients with von Hippel Lindau disease but have infrequently been reported in patients without this syndrome. A 35-year-old woman with a history of neurofibromatosis type 1 presented to our care with visual loss and headaches. Magnetic resonance imaging of the brain demonstrated an 8-mm cystic, contrast-enhancing lesion abutting the optic chiasm and optic tracts. The patient's endocrine profile was unremarkable. The tumor was resected using an endoscopic expanded transsphenoidal approach. Pathologic evaluation was consistent with hemangioblastoma. Postoperatively, the patient experienced an improvement in her visual symptoms. Hemangioblastoma should be included in the differential of sellar/suprasellar mass lesions, particularly in patients with von Hippel Lindau disease. Small suprasellar lesions may be safely and effectively removed using an expanded transsphenoidal approach.